Familial Primary Systemic Amyloidosis: An Experimental, Genetic and Clinical Study1

Rukavina, John G.; Block, Walter D.; Curtis, Arthur C.

doi:10.1038/jid.1956.84

Cited by 27 publications

(3 citation statements)

References 10 publications

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“…In one study of FAP, autonomic neuropathy ultimately developed in 70% of patients [14] and impotence is frequent and early. Type II FAP was first described in a Swiss family living in Indiana [15]. These patients did not develop symptoms until the fourth or fifth decade of their lives.…”

Section: Differential Diagnosismentioning

confidence: 98%

Primary Systemic Amyloid Neuropathy

Bajwa

Kelly

2010

Amyloidosis

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Primary systemic amyloidosis (PSA) is a plasma cell dyscrasia. Organ damage is caused by deposition of amyloid, derived from monoclonal light chains, in tissues including nerves. PSA damages tissues and leads to combinations of neuropathy and renal, cardiac, and liver failure. The neuropathy of PSA is relatively stereotyped with damage to small and autonomic nerve fibers and superimposed carpal tunnel syndrome. The disease is progressive and mortality is high, especially when there is early involvement of vital organs. Treatment in the past was relatively ineffective. However, with the advent of peripheral blood stem cell transplantation and chemotherapy in selected patients, prolonged survival is possible if the weight of organ damage at onset of treatment is not too high. Thus, early detection is paramount.

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Section: Differential Diagnosismentioning

confidence: 98%

Primary Systemic Amyloid Neuropathy

Bajwa

Kelly

2010

Amyloidosis

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“…A family with amyloidosis who were of Swiss origin and residing in Indiana was reported in 1956 (Rukavina et al , 1956). The first manifestation is carpal tunnel syndrome followed by peripheral neuropathy involving the lower extremities.…”

Section: Familial Amyloidosismentioning

confidence: 99%

Amyloidosis: a convoluted story

2001

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“…The family was extensively investigated by Rukavina et al (1956a;1956b), and later reviewed by Jackson and Block (1970). Out of 66 members investigated, 29 were found to be affected.…”

mentioning

confidence: 99%

Genetic factors in amyloidosis.

Thomas¹

1975

Journal of Medical Genetics

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Familial Primary Systemic Amyloidosis: An Experimental, Genetic and Clinical Study1

Cited by 27 publications

References 10 publications

Primary Systemic Amyloid Neuropathy

Primary Systemic Amyloid Neuropathy

Amyloidosis: a convoluted story

Genetic factors in amyloidosis.

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